Anxiety, distress, and pain in pediatric urodynamics.

AIMS: No one has assessed urodynamic studies (UDS) to determine those steps that elicit the greatest anxiety, distress, and pain in children. We sought to systematically evaluate a child's UDS experience to mollify these reactions. METHODS: Prospective study involving children aged ≥5 undergoing UDS over a 6-month period (from 10 December 2018 to 22 May 2019). Upon arrival, patients completed a visual analog scale for anxiety (VAS-A, 0-10) about the upcoming procedure. A research assistant assessed the patient's behavior during each major step of UDS using a validated brief behavioral distress scale. Nursing staff also obtained patients' pain ratings (0-10) for these key elements. Immediately after UDS, each child completed a posttest VAS-A along with a survey about the UDS experience. RESULTS: A total of 76 UDS were observed; almost half included sphincter needle electromyography (EMG). Mean patient VAS-A scores were 2.3 before UDS, compared to 0.8 afterward (P < .001). The highest proportion of distressful behaviors were observed during EMG needle (31%) and urethral catheter (29%) insertion, in agreement with the highest mean pain scores of 3.2 and 2.7, respectively. Fifty-four percent of children reported not being completely aware of what was going to happen before the procedure and 50% of those patients exhibited at least one interfering or potentially interfering behavior. Similarly, 60% of children with no prior history of UDS exhibited at least one interfering or potentially interfering behavior. CONCLUSIONS: EMG needle and urethral catheter placement, initial urodynamic testing and not knowing what to expect were associated with greater pain and distress during pediatric UDS.

Risk factors for non-adherence to disease-modifying therapy in pediatric multiple sclerosis.

BACKGROUND: Adherence to disease-modifying therapies (DMTs) in pediatric multiple sclerosis (MS) is not well understood. We examined the prevalence and risk factors for poor adherence in pediatric MS. METHODS: This cross-sectional study recruited youth with MS from 12 North American pediatric MS clinics. In addition to pharmacy-refill data, patients and parents completed self-report measures of adherence and quality of life. Additionally, patients completed measures of self-efficacy and well-being. Factor analysis and linear regression methods were used. RESULTS: A total of 66 youth (mean age, 15.7 years) received MS DMTs (33% oral, 66% injectable). Estimates of poor adherence (i.e. missing >20% of doses) varied by source: pharmacy 7%, parent 14%, and patient 41%. Factor analysis yielded two composites: adherence summary and parental involvement in adherence. Regressions revealed that patients with better self-reported physical functioning were more adherent. Parents were more likely to be involved in adherence when their child had worse parent-reported PedsQL School Functioning and lower MS Self-Efficacy Control. Oral DMTs were associated with lesser parental involvement in adherence. CONCLUSION: Rates of non-adherence varied by information source. Better self-reported physical functioning was the strongest predictor of adherence. Parental involvement in adherence was associated with worse PedsQL School Functioning and lower MS Self-Efficacy-measured confidence in controlling MS.

Correction to: Impact of an electronic monitoring device and behavioural feedback on adherence to multiple sclerosis therapies in youth: results of a randomized trial.

The clinicaltrials.gov identifying number for the article titled "Impact of an electronic monitoring device and behavioral feedback on adherence to multiple sclerosis therapies in youth: results of a randomized trial" is NCT02234713 (https://clinicaltrials.gov/ct2/show/NCT02234713).

Impact of an electronic monitoring device and behavioral feedback on adherence to multiple sclerosis therapies in youth: results of a randomized trial.

PURPOSE: To report the results of a randomized controlled trial using an electronic monitoring device (EM) plus a motivational interviewing (MI) intervention to enhance adherence to disease-modifying therapies (DMT) in pediatric MS. METHODS: Fifty-two youth with MS (16.03 ± 2.2 years) were randomized to receive either MI (n = 25) (target intervention) or a MS medication video (n = 27) (attention control). Primary endpoint was change in adherence. Secondary outcomes included changes in quality of life, well-being and self-efficacy. Random effects modeling and Cohen's effect size computation evaluated intervention impact. RESULTS: Longitudinal random effect models revealed that the MI group decreased their EM adherence (GroupxTime interaction = -0.19), while increasing frequency of parental DMT reminder (26.01)/administration (11.69). We found decreased EM use in the MI group at 6 months (Cohen's d = -0.61), but increased pharmacy refill adherence (d = 0.23). Parental reminders about medication increased in MI subjects vs controls (d = 0.59 at 3 months; d = 0.70 at 6 months). We found increases in self-reported adherence (d = 0.21) at 3 but not 6 months, fewer barriers to adherence at three (d = -0.58) and six months (d = -0.31), better physical (d = 0.23 at 3 months; d = 0.45 at 6 months), emotional (d = 0.25 at 3 months) and self-efficacy function (d = 0.55 at 3 months; 0.48 at 6 months), but worse well-being, including self-acceptance (d = -0.53 at 6 months) and environmental mastery (d = -0.42 at 3 and 6 months) in intervention as compared to control patients. CONCLUSIONS: Participants receiving MI + EM experienced worsening on objective measures of adherence and increased parental involvement, but improved on some self- and parent-reported measures. MI participants reported improvements in quality of life and self-efficacy, but worsened well-being.

Health related quality of life in patients with bladder exstrophy: a call for targeted interventions.

PURPOSE: Research on health related quality of life in patients with bladder exstrophy has demonstrated mixed results compared to population samples. Few studies have had a comparison group with a urological disorder and none correlated body image to health related quality of life. We compared health related quality of life in patients with bladder exstrophy to that in patients with kidney stones and correlated body image to health related quality of life in the bladder exstrophy population. MATERIALS AND METHODS: Participants included 24 patients with kidney stones and 24 with bladder exstrophy. Patient age was 8 to 25 years. Participants completed the PedsQL (Pediatric Quality of Life Inventory) and the 25-item multiple choice UBIQ (Urological Body Image Questionnaire), which was created for the current study. RESULTS: Compared to patients with kidney stones those with bladder exstrophy indicated significantly higher scores on physical functioning (p = 0.0005) and overall health related quality of life (p = 0.02) in the 8 to 17-year-old cohort. When health related quality of life was compared to body image in patients with bladder exstrophy, lower social scores were associated with those who worried about friends finding out about the condition (p = 0.01) and about having romantic relationships (p = 0.003). Lower social (p = 0.006) and emotional (p = 0.009) functioning scores were associated with patients who thought that they were less likely to have romantic relationships. CONCLUSIONS: The finding that patients with bladder exstrophy had a better physical functioning score than those with kidney stones is not surprising, given the morbidity of active stone disease. The negative correlations with body image in the bladder exstrophy population may be due to the possibility of others discovering the condition and the perceived likelihood of not having romantic relationships.

Routine behavioral and mental health screening in young children with type 1 diabetes mellitus.

BACKGROUND: The American Diabetes Association and International Society for Pediatric and Adolescent Diabetes recommend that providers of diabetes care receive training in the recognition of psychosocial problems related to diabetes. OBJECTIVE: To report the results of routine behavioral/mental health screening for children with type 1 diabetes mellitus (T1D) seen in a multidisciplinary pediatric diabetes program. SUBJECTS AND METHODS: This was a cross-sectional study of children with T1D ages 4-11 years, who underwent behavioral/mental health screening as part of their diabetes care. Screening utilized the Strengths and Difficulties Questionnaire (SDQ) Parent Proxy Version, and scores were reviewed by a social worker. SDQ scale and total difficulties scores were compared by gender, visit type, age, T1D duration, and HbA1c. Scores were also compared to age-appropriate normative data for children in United States of America (US). RESULTS: SDQ Parent Proxy Version total difficulties and scale scores did not differ by patient or visit characteristics. Compared with normative data for US children, a greater proportion of children with T1D ages 4-7 and 8-10 years had borderline/abnormal scores on the emotional symptoms scale (p = 0.01 and p = 0.03, respectively), suggesting risk for psychological disorders, such as anxiety and depression. CONCLUSIONS: Our findings suggest that children less than 11 years old with T1D may have greater emotional symptoms as compared to their age-matched healthy peers. Pediatric diabetes care providers, with access to mental health services, should consider incorporating routine behavioral/mental health screening for children less than 12 years old in their practice.

Relationship among chelator adherence, change in chelators, and quality of life in thalassemia.

PURPOSE: Thalassemia, a chronic blood disease, necessitates life-long adherence to blood transfusions and chelation therapy to reduce iron overload. We examine stability of health-related quality of life (HRQOL) in thalassemia and adherence to chelation therapy over time, especially after changes in chelator choice. METHODS: Thalassemia Longitudinal Cohort participants in the USA, UK, and Canada completed the SF-36v2 (ages 14+) and the PF-28 CHQ (parents of children <14 years). Chelation adherence was defined as self-reported percent of doses administered in the last 4 weeks. RESULTS: Two hundred and fifty-eight adults/adolescents (mean 29.7 years) and 133 children (mean 8.5 years) completed a mean of 2.8-years follow-up. Children made few chelator changes, whereas a mean of 2.2 changes was observed among the 37% of adults/adolescents who made chelator changes, mainly due to patient preference or medical necessity. Physical HRQOL improved among those with lower iron burden (better health status) at baseline who made a single change in chelator, but declined among participants with multiple changes and/or high iron burden (worse health status). Mental health improved among participants with lower iron burden, but iron overload was negatively associated with social functioning. Adherence did not significantly change over follow-up except for an increase after a change from deferoxamine (DFO) infusion to oral deferasirox (p = 0.03). Predictors of lower adherence for adults/adolescents at follow-up included side effects, smoking, younger age, problems preparing DFO, increased number of days per week DFO prescribed, and lower physical quality of life . CONCLUSIONS: Strategies to balance medical needs with family, work, and personal life may assist in adherence.

A survey to assess body and self-image in individuals with bladder exstrophy: a call for psychosocial support.

PURPOSE: Although research in patients with urological conditions indicates that functional limitations, pain, limited mobility and social restrictions can impact self-image, to our knowledge the influence of the visibility of the condition has not been examined. We assessed body image and self-image at school age through young adulthood in patients with bladder exstrophy compared to patients with kidney stones. MATERIALS AND METHODS: Patients diagnosed with bladder exstrophy and kidney stones completed a 25-item multiple choice Urological Body Image Questionnaire for ages 13 to 25 years, which was created for the current study. It consists of questions on physical appearance, avoidance behaviors, urological condition disclosure, romantic relationships and future expectations. A modified Urological Body Image Questionnaire for patients 8 to 12 years old was created by removing questions on relationships and future expectations. RESULTS: A total of 24 patients with bladder exstrophy and 24 with kidney stones 8 to 25 years old completed the questionnaire. Compared to patients with kidney stones, patients with bladder exstrophy were significantly more likely to avoid changing clothes in front of peers and to not tell friends about the condition (each p = 0.0001). Patients with bladder exstrophy were also significantly more likely to think that the condition would prevent having future romantic relationships (p = 0.002) and children (p = 0.003). CONCLUSIONS: Differences in the visibility and privacy of the 2 conditions likely explain the discrepancy between the groups. Findings may call for an individual approach to assessing issues related to self-esteem and identity in patients with bladder exstrophy and may highlight the importance of receiving psychosocial support.

Causal attributions of cleft lip and palate across cultures.

Objective : To describe and compare the causal beliefs associated with cleft lips and/or palates across several different countries. Design : Cross-sectional survey. Setting : Operation Smile surgery screenings in six developing countries. Participants : Two hundred seventy-nine adult patients and parents of children with cleft lips and/or palates in Kenya, Russia, Cambodia, India, Egypt, and Peru. Interventions : In person interviews were conducted with interpreters. Main Outcome Measure : As part of a larger study, a semistructured questionnaire was created to explore cleft perceptions, belief systems that affect these perceptions, and social reactions to individuals with clefts. Results : Causal attributions were grouped by category (environment, self-blame, supernatural, chance, unknown, or other) and type of locus of control (external, internal, or unknown). Results indicate significant difference by country for both causal attribution category (P < .001) and type (P < .001). This difference was maintained in multivariate analyses, which controlled for differences by demographic variables between countries. Conclusions : This study provides evidence that causal attributions for clefts are influenced by culture. As harmful beliefs about cause may continue to impact affected individuals and their families even after a repair, it is insufficient to provide surgical care alone. Care of the entire person must include attempts to change misinformed cultural beliefs through educating the broader community.

Beliefs about chelation among thalassemia patients.

BACKGROUND: Understanding patients' views about medication is crucial to maximize adherence. Thalassemia is a congenital blood disorder requiring chronic blood transfusions and daily iron chelation therapy. METHODS: The Beliefs in Medicine Questionnaire (BMQ) was used to assess beliefs in chelation in thalassemia patients from North America and London in the Thalassemia Longitudinal Cohort (TLC) of the Thalassemia Clinical Research Network (TCRN). Chelation adherence was based on patient report of doses administered out of those prescribed in the last four weeks. RESULTS: Of 371 patients (ages 5-58y, mean 24y), 93% were transfused and 92% receiving chelation (26% deferoxamine (DFO; a slow subcutaneous infusion via portable pump), 63% oral, 11% combination). Patients expressed high "necessity" for transfusion (96%), DFO chelation (92%) and oral chelation (89%), with lower "concern" about treatment (48%, 39%, 19% respectively). Concern about oral chelation was significantly lower than that of DFO (p<0.001). Self-reported adherence to chelation was not associated with views about necessity or concerns, but negatively correlated with perceived sensitivity to DFO (Sensitive Soma scale; r=-0.23, p=0.01) and side effects of oral chelation (r=-0.14, p=0.04). High ferritin iron levels, potentially indicating lower adherence, were found in 41% of patients reporting low necessity of oral chelation compared to 24% reporting high necessity (p=0.048). Concerns about treatment were associated with lower quality of life and more symptoms of anxiety and depression. CONCLUSIONS: Despite their requirement for multimodal therapy, thalassemia patients have positive views about medicine, more so than in other disease populations. Patients may benefit from education about the tolerability of chelation and strategies to effectively cope with side effects, both of which might be beneficial in lowering body iron burden. CLINICALTRIALS.GOV IDENTIFIER: NCT00661804.

Iron chelation adherence to deferoxamine and deferasirox in thalassemia.

The Thalassemia Clinical Research Network collected adherence information from 79 patients on deferoxamine and 186 on deferasirox from 2007 to 2009. Chelation adherence was defined as percent of doses administered in the last 4 weeks (patient report) out of those prescribed(chart review). Chelation history since 2002 was available for 97 patients currently on deferoxamine and 217 on deferasirox, with crude estimates of adherence from chart review. Self-reported adherence to both deferoxamine and deferasirox were quite high, with slightly higher adherence to the oral chelator (97 vs. 92%). Ninety percent of patients on deferasirox reported at least 90% adherence, compared with 75% of patients on deferoxamine. Adherence to both chelators was highest in children, followed by adolescents and older adults.Predictors of lower deferoxamine adherence were smoking in the past year, problems sticking themselves (adults only), problems wearing their pump, and fewer transfusions in the past year. Predictors of lower deferasirox adherence were bodily pain and depression. Switching chelators resulted in increased adherence, regardless of the direction of the switch, although switching from deferoxamine to deferasirox was far more common. As adherence to deferoxamine is higher than previously reported, it appears beneficial for patients to have a choice in chelators.

"Ready SIM Go": An Adapted Simulation "Service Line" for Patients and Caregivers.

SUMMARY STATEMENT: Changing healthcare systems and efforts to decrease medical costs have resulted in many more patients and caregivers (PCs) needing to manage life-sustaining therapies on their own. Given the potential for underpreparedness to result in increased morbidity and mortality, developing safe and efficient homecare training methods for PCs is essential. Medical simulation with PCs is an underused tool, which has the potential to decrease anxiety and increase preparedness through opportunities for repeated practice of medical procedures within a safe, controlled environment. This article describes the development of a hospital-based simulation service line for PCs, leveraging lessons learned from training for 250 patients and 450 caregivers in 14 different departments and subspecialties, and explains adaptations of standard simulation methods necessary for safe and effective use of simulation with this unique population. Process blueprint, examples of specific programs, as well as feedback from participants and clinicians are included.

Aligning Family-Clinician Expectations During Pediatric Surgical Informed Consent: Development and Implementation of an Innovative Communication Skills Workshop.

INTRODUCTION: Aligning expectations during the informed consent process before a child's surgery is an important element of good communication that benefits both surgical staff and families. We developed and evaluated a 2-hour pilot interprofessional workshop to improve the communication and relational skills of pediatric surgeons and nurse practitioners. METHODS: Focus groups with families identified key challenges in the process of informed consent. An interprofessional team, including parents whose children had experienced complex surgeries, developed the workshop collaboratively. A realistic simulation with professional actors portraying parents allowed surgical staff to practice communication skills and receive feedback about the parent perspective. Participants completed a postworkshop evaluation to determine whether the workshop met its objectives and whether they would change practice. RESULTS: Five key themes identified for the workshop included customize communication; align expectations; share clinical uncertainty; recognize/attend to emotions; and identify team members. Thirty-five clinicians participated in a workshop, and 89% completed evaluations. Three-quarters reported the learning to be valuable, and 64% were likely to change practice. Eighty-seven percent would recommend the workshop to other colleagues, and 58 to 74% felt more prepared to achieve each of eight specific skills. DISCUSSION: An innovative workshop for pediatric surgical practitioners to align family-clinician expectations can help improve clinician communication skills and comfort with informed consent. Keys to workshop development included involving parents to identify themes and participate as workshop co-faculty; enlisting leadership and recruiting surgical champions; and using pre-existing meetings to ease scheduling challenges of busy practitioners. Booster sessions may facilitate the desired cultural changes.

SIMDiscovery: a simulation-based preparation program for adolescents undergoing spinal fusion surgery.

PURPOSE: Spinal fusion surgery is associated with high levels of stress and anxiety for patients and their caregivers. Medical simulation has demonstrated efficacy in improving preparedness, knowledge, and overall experience prior to other medical procedures. The current study examines the utility of a multi-faceted preparation program (SIMDiscovery) using simulation techniques to reduce anxiety and increase preparedness among patients undergoing spinal fusion surgery and their caregivers. METHODS: Participants attended SIMDiscovery where they received hands-on preparation about what to expect before, during, and after their surgery. Anxiety, preparedness, and knowledge about the procedure were assessed pre- and post-participation using self-report measures. Participants also completed a questionnaire at their first post-operative medical appointment. Differences from pre to post and between patients and caregivers were calculated with paired and independent sample t-tests. RESULTS: Participants included 22 patients and 29 caregivers. Post-SIMDiscovery, both groups demonstrated increased knowledge for the surgical process and lower state anxiety. Patients reported increased feelings of preparedness in all areas while caregivers reported increased feelings of preparedness in most areas. Families continued to report positive impact of the program 30 days after surgery; however, they also identified areas where they desired increased preparation. CONCLUSIONS: SIMDiscovery increased patients' and caregivers' knowledge regarding spinal fusion surgery and helped them feel less anxious and more prepared regarding most aspects of the surgical process. These changes were generally maintained throughout the post-operative period. Participants identified areas for increased preparation, highlighting the importance of continuing to adapt programs based on patient and family feedback. LEVEL OF EVIDENCE: Level III.

Symptoms of depression and anxiety in patients with thalassemia: prevalence and correlates in the thalassemia longitudinal cohort.

Thalassemia is an inherited blood disorder that requires lifelong adherence to a complicated and burdensome medical regimen which could potentially impact emotional functioning of patients. The importance of understanding and promoting healthy emotional functioning is crucial not only to psychological well-being, but also to physical health as it has been shown to impact adherence to medical regimens [1-4]. The current study aimed to [1] determine the prevalence of depressive and anxiety symptoms in adolescent and adult patients with thalassemia; and [2] explore possible demographic, medical, and psychosocial correlates of these symptoms in 276 patients (14-58 years old, M age = 27.83; 52% female). Overall, most patients did not report experiencing significant symptoms of anxiety and depression (33% of participants indicated experiencing symptoms of anxiety and 11% symptoms of depression). Females and older patients were more likely to experience these symptoms than males and younger patients. Symptoms of anxiety and depression were positively associated with self-report of difficulty with adherence and negatively associated with quality of life. Given these findings, regular screening for anxiety and depression symptoms could help to identify at-risk individuals to provide them with appropriate psychological support with the goal of improving both emotional and physical health.

Oral chelation: should it be used with young children?

Deferasirox, the only oral iron chelator approved in the US and Canada, achieved a pediatric label indication down to 2 years of age. FDA-mandated post-marketing safety surveillance in children under age 6 is ongoing. We wish to raise a non-safety-related concern with deferasirox chelation in very young children (2-5 years old). Specifically, the circumstances required for reliable daily ingestion of deferasirox do not mesh well with the developmental characteristics common in young children, which may limit adherence and cause parental distress. We review developmental challenges associated with oral chelation in this age group and provide suggestions to improve adherence in this population.

Stress and coping of parents of young children diagnosed with bladder exstrophy.

PURPOSE: Previous studies have examined the psychological impact that living with bladder exstrophy has on patients. However, little is known about how parents of children diagnosed with this condition are affected. We examine how parents caring for children diagnosed with bladder exstrophy are impacted. An increased understanding of the stressors these parents face may lead to the development of appropriate parenting interventions, which may ultimately affect psychosocial and health outcomes in the child. MATERIALS AND METHODS: All parents of children 10 years and younger treated for bladder exstrophy at our institution were selected from a centralized database. A total of 20 parents (65% of the eligible population) completed standardized questionnaires assessing pediatric specific parenting stress (Pediatric Inventory for Parents) and coping (Ways of Coping Questionnaire). RESULTS: Parents identified several common stressors (eg worrying about the long-term impact of the illness, helping the child with his/her hygiene needs) and overall reported using adaptive ways of coping (ie planful problem solving, seeking social support, positive reappraisal). However, when they experienced increased stress they reported using more nonadaptive ways of coping (ie escape/avoidance and distancing). CONCLUSIONS: Overall the findings of our study suggest that parents of children diagnosed with bladder exstrophy experience a significant amount of stress. In fact, parents in our study indicated experiencing similar frequency and difficulty of stress compared to parents of the same aged children diagnosed with type 1 diabetes. Increased stress can have negative consequences for parents and children. Future directions and implications of these findings are discussed.

Stress among fathers of young children with type 1 diabetes.

Although fathers' stress has been shown to have important implications for children's health and well-being, few studies of children with Type 1 diabetes have considered paternal parenting stress. The current study contributes to the literature by exploring correlates of fathers' pediatric parenting stress in a sample of young children with Type 1 diabetes. Forty-three fathers of children 2-6 years old with Type 1 diabetes completed self-report questionnaires examining pediatric parenting stress, child behavior, participation in diabetes management tasks, and parental psychological resources. Results of multiple regression show that fathers' pediatric parenting stress is positively associated with state anxiety and mother-reported difficult child behavior. These findings suggest that fathers may experience parenting stress differently than mothers, and that their experiences may have implications not only for their own level of anxiety, but also for their children's behavior.

Parental anxiety and depression associated with caring for a child newly diagnosed with type 1 diabetes: opportunities for education and counseling.

OBJECTIVE: To examine demographic and clinical characteristics, such as pediatric parenting stress and self-efficacy for diabetes care, of parents of children newly diagnosed with type 1 diabetes that are associated with parental anxiety and depression. METHODS: 102 parents reported on their levels of depression (CESD), state anxiety (STAI), pediatric parenting stress (PIP), and self-efficacy for diabetes care (SED) within 4 weeks of their child's diagnosis with type 1 diabetes. Data were analyzed using hierarchical multiple regression. RESULTS: Parents' scores in the clinical range for depression and anxiety were associated with increased frequency and difficulty of pediatric parenting stress, and there was a trend for depression to be related to lower self-efficacy for diabetes care. The association of female gender with anxiety and depression was partially mediated by more frequent pediatric parenting stress. CONCLUSION: Parents of children newly diagnosed with type 1 diabetes are at risk for experiencing anxiety and depression, related, in part, to their experiences of pediatric parenting stress. PRACTICE IMPLICATIONS: Providers and educators should be aware of the risk for depression and anxiety in parents and should work to decrease pediatric parenting stress, increase self-efficacy, and refer parents who are experiencing significant anxiety or depression following their child's diagnosis to a mental health specialist.